Rare Presentation of Right Adrenal Mass : Extramedullary Haematopoiesis in a Patient with Thalassaemia Intermedia

Poh Shean Wong; Lit Sin  Yong; Nor Afidah  Karim; Ee Leng  Gan; See Guan  Toh; Noor Lita  Adam

doi:10.15605/jafes.036.01.04

Authors

Poh Shean Wong Hospital Tuanku Ja'afar Seremban, Malaysia https://orcid.org/0000-0002-4686-5515
Lit Sin Yong Hospital Tuanku Ja'afar Seremban, Malaysia https://orcid.org/0000-0001-8873-7768
Nor Afidah Karim Hospital Tuanku Ja’afar Seremban, Malaysia
Ee Leng Gan Hospital Tuanku Ja’afar Seremban, Malaysia https://orcid.org/0000-0002-0270-068X
See Guan Toh Hospital Tuanku Ja’afar Seremban, Malaysia https://orcid.org/0000-0002-1162-7605
Noor Lita Adam Hospital Tuanku Ja’afar Seremban, Malaysia https://orcid.org/0000-0001-8679-3483

DOI:

https://doi.org/10.15605/jafes.036.01.04

Keywords:

Adrenal mass, Adrenal incidentaloma, Extramedullary hematopoiesis, Thalassaemia, Congenital haemolytic disorder

Abstract

Extramedullary hematopoiesis (EMH) is a rare cause of adrenal mass. We present a 44-year-old woman who has thalassaemia intermedia, referred to Endocrinology clinic for huge adrenal mass. Along with a paraspinal lesion discovered in this patient, the leading diagnosis was EMH. The patient was treated with hypertransfusion and hydroxyurea, which led to a reduction in the size of the right adrenal mass and paraspinal mass.

This case highlights the challenges in managing this rare condition. Although EMH is a rare cause of adrenal mass, the diagnosis must be considered in any patient with a history of a congenital hemolytic disorder, to avoid unnecessary surgical procedures.

Downloads

Download data is not yet available.

Author Biographies

Poh Shean Wong, Hospital Tuanku Ja'afar Seremban, Malaysia

Endocrine Fellow. Endocrinology Unit, Department of Medicine

Lit Sin Yong , Hospital Tuanku Ja'afar Seremban, Malaysia

Endocrinology Unit, Department of Medicine

Nor Afidah Karim , Hospital Tuanku Ja’afar Seremban, Malaysia

Endocrinology Unit, Department of Medicine

Ee Leng Gan, Hospital Tuanku Ja’afar Seremban, Malaysia

Haematology Unit, Department of Medicine

See Guan Toh , Hospital Tuanku Ja’afar Seremban, Malaysia

Haematology Unit, Department of Medicine

Noor Lita Adam , Hospital Tuanku Ja’afar Seremban, Malaysia

Endocrinology Unit, Department of Medicine

References

Harb Perspect Med. 2013;3(2):a011775. PMID: 23378598. PMCID:PMC3552345. https://doi.org/10.1101/cshperspect.a011775.

Weatherall DJ. The evolving spectrum of the epidemiology of thalassemia. Hematol Oncol Clin North Am. 2018;32(2):165-75. PMID:29458724. https://doi.org10.1016/j.hoc.2017.11.008.

Ibrahim HM, Muda Z, Othman IS, et al. Observational study on the current status of thalassaemia in Malaysia: A report from the Malaysian Thalassaemia Registry. BMJ Open. 2020;10(6):e037974.

PMID: 32601117. PMCID: PMC7328811. https://doi.org/10.1136/bmjopen-2020-037974.

Rivella S. The role of ineffective erythropoiesis in non-transfusiondependent thalassemia. Blood Rev. 2012;26 Suppl 1(0 1):S12-5. PMID: 22631035. PMCID: PMC3697110. https://doi.org/10.1016/S0268-960X(12)70005-X.

Sohawon D, Lau KK, Lau T, Bowden DK. Extra-medullary haematopoiesis: A pictorial review of its typical and atypical locations. J Med Imaging Radiat Oncol. 2012;56(5):538-44. PMID: 23043573.

https://doi.org/10.1111/j.1754-9485.2012.02397.x.

Arkadopoulos N, Kyriazi M, Yiallourou AI, et al. A rare coexistence of adrenal cavernous hemangioma with extramedullary hemopoietic tissue: A case report and brief review of the literature. World J Surg Oncol. 2009;7:13. PMID: 19193247. PMCID: PMC2647540. https://doi.org/10.1186/1477-7819-7-13.

Boucher A, Puech P, Kharroubi D, et al. Renal extramedullary hematopoietic tumor revealing a hereditary spherocytosis in an adult patient. Rev Med Interne. 2015;36(12):848–53. PMID: 25795467.

https://doi.org/10.1016/j.revmed.2015.02.007.

Karami H, Kosaryan M, Taghipour M, Sharifian R, Aliasgharian A, Motalebi M. Extramedullary hematopoiesis presenting as a right adrenal mass in a patient with beta thalassemia. Nephrourol

Mon. 2014;6(5):e19465. PMID: 25695031. PMCID: PMC4318012. https://doi.org/10.5812/numonthly.19465.

Azarpira N , Esfahani MH, Paydar S . Extramedullary hematopoiesis in adrenal gland. An uncommon cause of adrenal incidentaloma in sickle cell disease. Iran J Pediatr. 2014;24(6):784-6. PMID: 26019789.

PMCID: PMC4442845.

Castelli R, Graziadei G, Karimi M, Cappellini MD. Intrathoracic masses due to extramedullary hematopoiesis. Am J Med Sci. 2004;328(5):299–303. PMID: 15545849. https://doi.org/10.1097/00000441-200411000-00012.

Lau HY, Lui DC, Ma JKF, Wong RWC. Sonographic features of adrenal extramedullary hematopoiesis. J Ultrasound Med 2011;30(5):706–13. PMID: 21527620. https://doi.org/10.7863/jum.2011.30.5.706.

Porcaro AB, Novella G, Antoniolli SZ, et al. Adrenal extramedullary hematopoiesis: Report on a pediatric case and update of the literature. Int Urol Nephrol. 2001;33(4):601-3. PMID: 12452605. https://doi.org/10.1023/a:1020578501167.

Chuang CK, Chu SH, Fang JT, Wu JH. Adrenal extramedullary hematopoietic tumor in a patient with beta-thalassemia. J Formos Med Assoc. 1998;97(6):431-3. PMID: 9650475.

Papavasiliou C, Gouliamos A, Deligiorgi E, Vlahos L, Cambouris T. Masses of myeloadipose tissue: Radiological and clinical considerations. Int J Radiat Oncol Biol Phys. 1990;19(4):985–93. PMID:2211267. https://doi.org/10.1016/0360-3016(90)90023-d.

Keikhaei B, Shirazi AS, Pour MM. Adrenal extramedullary hematopoiesis associated with β-thalassemia major. Hematol Rep. 2012;4(2):e7. PMID: 22826797. PMCID: PMC3401136. https://doi.org/10.4081/hr.2012.e7.

Wat NM, Tse KK, Chan FL, Lam KS. Adrenal extramedullary haemopoiesis: Diagnosis by a non-invasive method. Br J Haematol. 1998;100(4):725–7. PMID: 9531340. https://doi.org/10.1046/j.1365-2141.1998.00619.x.

Calhoun SK, Murphy RC, Shariati N, Jacir N, Bergman K. Extramedullary hematopoiesis in a child with hereditary spherocytosis: An uncommon cause of an adrenal mass. Pediatr Radiol. 2001;31(12):879–81. PMID: 11727025. https://doi.org/10.1007/s002470100011.

Bastounis EA, Karayiannakis AJ, Anapliotou ML, Nakopoulou L, Makri GG, Papalambros EL. Incidentalomas of the adrenal gland: Diagnostic and therapeutic implications. Am Surg. 1997;63(4):356-60. PMID: 9124759.

Asnani M, Char G, Reid M, Nanton PM. Intrathoracic and pelvic extramedullary hematopoiesis in sickle cell disease: A case report. West Indian Med J. 2007;56(6):540-3. PMID: 18646500,

Orphanidou-Vlachou E, Tziakouri-Shiakalli C, Georgiades CS. Extramedullary hemopoiesis. Semin Ultrasound CT MR. 2014;35(3):255–62. PMID: 24929265. https://doi.org/10.1053/j.sult.2013.12.001.

De Klippel N, Dehou MF, Bourgain C, Schots R, De Keyser J, Ebinger G. Progressive paraparesis due to thoracic extra-medullary hematopoiesis in myelofibrosis. Case report. J Neurosurg. 1993;79(1):

–7. PMID: 8315451. https://doi.org/10.3171/jns.1993.79.1.0125.

Cone SM. Bone marrow occurring in intercostal veins. JAMA 1925;84: 1732–3.

Oustwani MB, Kurtides ES, Christ M, Ciric I. Spinal cord compression with paraplegia in myelofibrosis. Arch Neurol. 1980;37(6):389–90. PMID: 7387474. https://doi.org/10.1001/archneur.1980.00500550091019.

Fassnacht M, Arlt W, Bancos I, et al. Management of adrenal incidentaloma: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study

of Adrenal Tumors. Eur Journal of Endocrinol. 2016;175(2):G1-34. PMID: 27390021. https://doi.org/10.1530/EJE-16-0467.

Banerji JS, Kumar RM, Devasia A. Extramedullary hematopoiesis in the adrenal: Case report and review of literature. Can Urol Assoc J. 2013;7(5-6):e436–8. PMID: 23826059. PMCID: PMC3699094.

https://doi.org/10.5489/cuaj.1389.

Al-Thani H, Al-Sulaiti M, El-Mabrok G, Tabeb A, El-Menyar A. Adrenal extramedullary haematopoiesis associated with betathalassaemia trait in an adult woman: A case report and review of literature. Int J Surg Case Rep. 2016;24:83-7. PMID: 27232291. PMCID:PMC4885136. https://doi.org/10.1016/j.ijscr.2016.05.024.

Tanner J., Malhotra S., El-Daly H., Godfrey E.M. Case 243: Extra-medullary hematopoiesis in an adrenal myelolipoma. Radiology 2017;284(1):292-6. PMID: 28628416. https://doi.org/10.1148/radiol.2017142993.

Kolev NH, Genov PP, Dunev VR, Stoykov BA. A rare case of extramedullary hematopoiesis in adrenal mass. Urol Case Rep. 2020;30:101120. PMID: 32055448. PMCID: PMC7005413. https://doi.org/10.1016/j.eucr.2020.101120.

Kannan S, Kulkarni P, Lakshmikantha A, Gadabanahalli K. Extramedullary haematopoiesis presenting as an adrenal mass. J Clin Diagn Res. 2017;11(3):TJ01. PMID: 28511479. PMCID: PMC5427405. https://doi.org./10.7860/JCDR/2017/25086.9448.

Sekar S, Burad D, Abraham A, Paul MJ. Adrenal incidentaloma caused by extramedullary haematopoiesis: conservative management is optimal. BMJ Case Rep. 2015;2015:bcr2015211014. PMID: 26392447. PMCID: PMC4577657. https://doi.org/10.1136/bcr-2015-211014.

King BF, Kopecky KK, Baker MK, Clark SA. Extramedullary hematopoiesis in the adrenal glands: CT characteristics. J Comput Assist Tomogr. 1987;11(2):342–3. PMID: 3819139. https://doi.org/10.1097/00004728-198703000-00030.

Kenney PJ, Wagner BJ, Rao P, Heffess CS. Myelolipoma: CT and pathologic features. Radiology. 1998;208(1):87-95. PMID: 9646797. https://doi.org/10.1148/radiology.208.1.9646797.

Decmann Á, Perge P, Tóth M, Igaz P. Adrenal myelolipoma: A comprehensive review. Endocrine. 2018;59(1):7-15. PMID: 29164520. https://doi.org/10.1007/s12020-017-1473-4.

Günther RW, Kelbel C, Lenner V. Real-time ultrasound of normal adrenal glands and small tumors. J Clin Ultrasound, 1984;12(4):211–7. PMID: 6427284. https://doi.org/10.1002/jcu.1870120408.

Dietrich CF, Wehrmann T, Hoffmann C, Herrmann G, Caspary WF, Seifert H. Detection of the adrenal glands by endoscopic or transabdominal ultrasound. Endoscopy. 1997;29(9):859–64. PMID:

https://doi.org/10.1055/s-2007-1004322.

Habibzadeh F, Yadollahie M, Haghshenas M. Extramedullary haematopoiesis in beta-thalassaemia. J Postgrad Med. 2005;51(1):45–6. PMID: 15793340.

Parsa K, Oreizy A. Nonsurgical approach to paraparesis due to extramedullary hematopoiesis: Report of two cases. J Neurosurg. 1995;82(4):657–60. PMID: 7897533. https://doi.org/10.3171/jns.1995.82.4.0657.

Cianciulli P, di Toritto TC, Sorrentino F, Sergiacomi L, Massa A, Amadori S. Hydroxyurea therapy in paraparesis and cauda equina syndrome due to extramedullary haematopoiesis in thalassaemia:

Improvement of clinical and haematological parameters. Eur J Haematol. 2000;64(6):426-9. PMID: 10901597. https://doi.org/10.1034/j.1600-0609.2000.9c165.x.

Cario H, Wegener M, Debatin KM, Kohne E. Treatment with hydroxyurea in thalassemia intermedia with paravertebral pseudotumors of extramedullary hematopoiesis. Ann Hematol. 2002;81(8):478–82. PMID: 12224008. https://doi.org/10.1007/s00277-002-0501-4.

Dore F, Pardini S, Gaviano E, et al. Recurrence of spinal cord compression from extramedullary hematopoiesis in thalassemia intermedia treated with low doses of radiotherapy. Am J Hematol.

;44(2):148. PMID: 8266922. https://doi.org/10.1002/ajh.2830440216.

Aliberti B, Patrikiou A, Terentiou A, Frangatous S & Papadimitrou A. Spinal cord compression due to extramedullary hematopoiesis in two patients with thalassemia: Complete regression with blood transfusion therapy. J Neurol. 2001;248(1):18– 22. PMID: 11266014. https://doi.org/10.1007/s004150170264.